Pulmonary embolism from right ventricular myxoma in a child with undiagnosed Carney complex.

Pediatric pulmonary embolism occurs in 8.6-57 per 100,000 hospitalised children. We report a novel case of bilateral pulmonary emboli in a child presenting with dyspnoea who was found to have large right ventricular myxoma and subsequent diagnosis of Carney complex. After resection of the right ventricular myxoma and bilateral pulmonary embolectomy, she had a full recovery and an excellent outcome.

Hospitalisations of children and adults with Hypoplastic Left Heart Syndrome in Texas from 2009 to 2019.

INTRODUCTION: Hypoplastic Left Heart Syndrome accounts for a significant proportion of CHD morbidity and mortality, despite improvements in care and improved survival. This study evaluates number of, reasons for, and trends in discharges of patients with hypoplastic left heart syndrome over 11 years in Texas. METHODS: The Texas Inpatient Discharge Dataset Public Use File captures almost all discharges in Texas and was reviewed from 2009 to 2019. Discharges of patients ≥5 years of age and diagnosis codes for Hypoplastic Left Heart Syndrome were included. The admitting and principle diagnoses were categorised and all discharges were evaluated for procedures performed. Descriptive and univariate statistical analyses were performed. RESULTS: A total of 1024 discharges were identified with a 16.9% annual increase over the study period. Median length of stay was 4 [IQR: 2-8] and there were 17 (1.7%) in-hospital mortalities with no differences across age groups. Seven (17.1%) discharges of patients 25+ years were uninsured, higher than other age groups (p < 0.001). The most common admitting diagnosis was CHD and 224 (21.9%) of discharges included a procedure, including 23 heart transplants. Discharges occurred from 67 different hospitals with 4 (6.0%) representing 71.4% of all discharges. CONCLUSIONS: Discharges of Hypoplastic Left Heart Syndrome have increased rapidly, particularly in the older age groups and were spread over a large number of hospitals. Further work is needed to understand the interplay between Hypoplastic Left Heart Syndrome and other conditions and care experiences that occur within the general population, which will become more common as this population ages and grows.

Trisomy 18: disparities of care and outcomes in the State of Texas between 2009 and 2019.

OBJECTIVE: To perform a statewide characteristics and outcomes analysis of the Trisomy 18 (T18) population and explore the potential impact of associated congenital heart disease (CHD) and congenital heart surgery. STUDY DESIGN: Retrospective review of the Texas Hospital Inpatient Discharge Public Use Data File between 2009 and 2019, analysing discharges of patients with T18 identified using ICD-9/10 codes. Discharges were linked to analyse patients. Demographic characteristics and available outcomes were evaluated. The population was divided into groups for comparison: patients with no documentation of CHD (T18NoCHD), patients with CHD without congenital heart surgery (T18CHD), and patients who underwent congenital heart surgery (T18CHS). RESULTS: One thousand one hundred fifty-six eligible patients were identified: 443 (38%) T18NoCHD, 669 (58%) T18CHD, and 44 (4%) T18CHS. T18CHS had a lower proportion of Hispanic patients (n = 9 (20.45%)) compared to T18CHD (n = 315 (47.09%)), and T18NoCHD (n = 219 (49.44%)) (p < 0.001 for both). Patients with Medicare/Medicaid insurance had a 0.42 odds ratio (95%CI: 0.20-0.86, p = 0.020) of undergoing congenital heart surgery compared to private insurance. T18CHS had a higher median total days in-hospital (47.5 [IQR: 12.25-113.25] vs. 9 [IQR: 3-24] and 2 [IQR: 1-5], p < 0.001); and a higher median number of admissions (n = 2 [IQR: 1-4]) vs. 1 [IQR: 1-2] and 1 [IQR: 1-1], (p < 0.001 for both). However, the post-operative median number of admissions for T18CHS was 0 [IQR: 0-2]. After the first month of life, T18CHS had freedom from in-hospital mortality similar to T18NoCHD and superior to T18CHD. CONCLUSIONS: Short-term outcomes for T18CHS patients are encouraging, suggesting a freedom from in-hospital mortality that resembles the T18NoCHD. The highlighted socio-economic differences between the groups warrant further investigation. Development of a prospective registry for T18 patients should be a priority for better understanding of longer-term outcomes.

Arrhythmias Requiring ECMO in Infants Without Structural Congenital Heart Disease.

Arrhythmias account for 55 per 100,000 patient evaluations in pediatric emergency departments. Most arrhythmias in children are amenable to medical management or cardioversion. Rarely, arrhythmias lead to significant hemodynamic instability requiring extracorporeal membrane oxygenation (ECMO) support. This study seeks to evaluate children under 1 year of age with a structurally normal heart requiring ECMO for an arrhythmia. This is a retrospective review of the Extracorporeal Life Support Organization Registry. All patients less than 1 year of age between 2009 and 2019 with a diagnosis of arrhythmia and without a diagnosis of structural heart malformation were included. Demographics, clinical characteristics, and outcomes were assessed with descriptive statistics and univariate and multivariable analyses. A total of 140 eligible patients were identified from the dataset. The most common arrhythmia was supraventricular tachycardia (SVT) in 70 (50%) patients. ECMO complications occurred in 106 (76.3%) patients and survival to discharge was achieved in 120 (85.7%) patients. In-hospital mortality was associated with neuromuscular blockade prior to ECMO [aOR 10.0 (95% CI 2.95-41.56), p < 0.001], neurologic ECMO complication [aOR 28.1 (95% CI 6.6-155.1), p < 0.001], and race with white race being protective [aOR 0.13, (95% CI 0.02-0.21), p = 0.002]. Similar survival and complication rates were found in subgroup analysis of SVT arrhythmias alone. Arrhythmias necessitating ECMO support in infants without structural congenital heart disease is a rare occurrence. However, survival to hospital discharge is favorable at greater than 85%. Given the favorable survival, earlier and more aggressive utilization of ECMO may result in improved outcomes.

Surgical Treatment of Acute Massive Pulmonary Embolism.

Massive pulmonary embolism (MPE) is a life-threatening condition. The management of MPE has changed over the course of the last few years. Since the emergence of thrombolytic therapy, only a few patients remain amenable for surgical treatment. Currently, surgical embolectomy is advised only in very specific indications. This chapter will review the background, history, indications, surgical technique and results of surgical pulmonary embolectomy in patients with MPE.

Valve-in-valve in the tricuspid position for a stenosed bioprosthesis.

The strategy of transcatheter valve-in-valve implantation into failing mitral and aortic bioprosthetic valves is a documented approach. It allows one to avoid performing a high-risk repeat cardiac surgery in elderly patients with multiple comorbidities. Tricuspid valve-in-valve implantation has been documented only a few times in the literature. We report the case of a 65-year-old woman with a failing bioprosthetic tricuspid valve who had undergone 3 prior open heart operations. We attempted a transatrial transcatheter approach and successfully deployed a 29-mm Edwards Sapien balloon-expandable bioprosthesis into a severely stenotic tricuspid bioprosthesis. This case demonstrates the technical feasibility and safety of this approach.

Duty Hours on Surgery Clerkship: From Compliance Nightmare to Leadership and Professional Development Opportunity.

OBJECTIVE: To evaluate the impact of an innovative leadership development initiative in the core surgery clerkship that addressed duty hours compliance and time-off requests. DESIGN: A combination of deductive and inductive analysis of medical student reflections written after rotating on Acute Care Surgery over 2 academic years (2019-2020 and 2020-2021) was performed. Reflections were part of criteria to receive honors and a prompt was given to discuss their experience in creating their own call schedules. We utilized a combined deductive and inductive process to identify predominant themes within the reflections. Once established, we quantitatively identified frequency and density of themes cited, along with qualitative analysis to determine barriers and lessons learned. SETTING: Dell Seton Medical Center, Dell Medical School at The University of Texas at Austin, a tertiary academic facility. PARTICIPANTS: There were 96 students who rotated on Acute Care Surgery during the study period, 64 (66.7%) of whom completed the reflection piece. RESULTS: We identified 10 predominant themes through the combined deductive and inductive processes. Barriers were cited by most students (n = 58, 91%), with communication being the most commonly discussed theme when cited with a mean 1.96 references per student. Learned leadership skills included: communication, independence, teamwork, negotiating skills, reflection of best practices by residents, and realizing the importance of duty hours. CONCLUSIONS: Transferring duty hour scheduling responsibilities to medical students resulted in multiple professional development opportunities while decreasing administrative burden and improving adherence to duty hour requirements. This approach requires further validation, but may be considered at other institutions seeking to improve the leadership and communication skills of its students, while improving adherence to duty hour restrictions.

Open and endoscopic fetal myelomeningocele surgeries display similar in-hospital safety profiles in a large, multi-institutional database.

BACKGROUND: Open intrauterine fetal myelomeningocele repair has demonstrated decreased ventriculoperitoneal shunting and improved motor outcomes despite maternal and fetal risks. Few data directly compare the safety of open vs endoscopic approaches. OBJECTIVE: This study aimed to analyze in-hospital maternal and fetal outcomes of pregnant patients undergoing open vs endoscopic fetal myelomeningocele repair using a large, multi-center database. STUDY DESIGN: This was a review of the Pediatric Health Information System database from October 1, 2015, to December 31, 2021. All patients who underwent open or endoscopic fetal myelomeningocele repair according to the International Classification of Diseases, Tenth Revision, were identified. Demographics, gestational age, and outcomes were analyzed. Descriptive and univariate statistics were used. RESULTS: A total of 378 pregnant patients underwent fetal myelomeningocele repair. The approach was endoscopic in 143 cases (37.8%) and open in 235 cases (62.2%). Overall postprocedural outcomes included no maternal in-hospital mortalities or intensive care unit admissions, a median length of stay of 4 days (interquartile range, 4-5), 14 cases (3.7%) of surgical and postoperative complications, 6 cases (1.6%) of intrauterine infections, 12 cases (3.2%) of obstetrical complications (including preterm premature rupture of membranes), 3 cases (0.8%) of intrauterine fetal demise, and 16 cases (4.2%) of preterm delivery. Compared with an open approach, the endoscopic approach occurred at a later gestational age (25 weeks [interquartile range, 24-25] vs 24 weeks [interquartile range, 24-25]; P<.001) and had an increased rate of intrauterine infection (6 [4.2%] cases vs 0 [0%] case; P=.002). There was no difference between approaches in the rates of surgical complications, obstetrical complications, intrauterine fetal demise, or preterm deliveries. CONCLUSION: Compared with an open approach, endoscopic fetal myelomeningocele repair displays a comparable rate of fetal complications, including intrauterine fetal demise and preterm delivery, and a similar in-hospital maternal safety profile despite an association with increased intrauterine infection.

Outcomes of thoracic aortic interventions in Marfan syndrome in the state of Texas over 11 years.

OBJECTIVES: Marfan syndrome is a heritable connective tissue disorder with significant aortopathy and conveys substantial cardiovascular morbidity. This study characterizes the mortality and morbidities of thoracic aortic interventions (TAI) in the Marfan syndrome population in the state of Texas from 2009 to 2019. METHODS: A retrospective review of the Texas Inpatient Discharge Dataset from 1 January 2009 to 31 December 2019. Discharges from acute care hospitals with a Marfan syndrome diagnosis by the International Classification of Diseases 9/10 codes and a procedure code for TAI were analysed utilizing descriptive, univariate and multivariable regression statistics. RESULTS: There were 4641 Marfan syndrome discharges identified, of whom 644 (13.9%) underwent TAI. Thoracic or thoraco-abdominal aortic dissection or rupture was noted in 223 (34.6%). Thirty-three (5.1%) had a concomitant coronary artery intervention. There were 30 (4.7%) in-hospital mortalities, 126 (19.6%) diagnoses of acute renal failure (ARF), 52 (8.1%) had mechanical ventilation >96 h and the median length of stay was 10 [interquartile range (IQR) 7-16] days. After adjustment, concomitant coronary artery intervention was associated with in-hospital mortality [odds ratio (OR) 3.69 [IQR 1.15-11.90], P = 0.029] and ARF (OR 2.66 [IQR 1.19-5.94], P = 0.017). Aortic dissections/ruptures were associated with ARF (OR 1.73 [IQR 1.14-2.63], P = 0.010), ventilation >96 h (OR 2.19 [IQR 1.21-3.97], P = 0.010), and 15% longer length of stay (95% confidence interval 2.4-29.1%, P = 0.038). CONCLUSIONS: TAI are frequent among the hospitalized Marfan Syndrome population. Concomitant coronary intervention is associated with increased risk of death and aortic dissections/ruptures are associated with increased morbidity. The high prevalence of aortic dissections/ruptures points to a potential target for improving imaging surveillance, adherence to treatment guidelines and preventative management of Marfan syndrome aortopathy.

Use of microaxial flow pumps in adolescents.

Objectives: The Impella 5.5 has been successfully used in the adult population; however, safety and efficacy data in patients aged less than 18 years are limited. Methods: Six pediatric patients, aged 13 to 16 years and weighing 45 to 113 kg, underwent axillary artery graft placement and attempted placement of the Impella 5.5 device at our institution between August 2020 and March 2023. Results: Indications for implantation were heart failure secondary to myocarditis (2), rejection of prior orthotopic heart transplant, idiopathic dilated cardiomyopathy (2), and heart failure after transposition of the great arteries repair. Placement was unsuccessful in a 13.8-year-old female patient due to prohibitively acute angulation of the right subclavian artery, and venoarterial extracorporeal membrane oxygenation cannulation was performed via the axillary graft. In 5 patients with successful Impella 5.5 placement, median duration of support was 13.5 days (range, 7-42 days). One experienced cardiac arrest secondary to coagulation-associated device failure, requiring temporary HeartMate3 implantation. Four patients were bridged to transplant; 3 patients received a transplant directly from Impella 5.5, and 1 patient received a transplant after HeartMate3. The final patient received the HeartMate3 on Impella day 42 and is awaiting transplant. Conclusions: Although exact size cutoffs and anatomy are still being determined, our experience provides a framework for use of the Impella 5.5 in adolescents.

Early diagnosis with sequencing and successful treatment of Bipolaris prosthetic valve endocarditis.

We present a case of a 48-year-old man with congenital bicuspid aortic valve, history of Ross procedure, prosthetic pulmonary valve and homograft with rapid molecular diagnosis and prompt surgical and medical treatment for Bipolaris fungal endocarditis with excellent outcome with early valve replacement, debridement, combination antifungal therapy, ongoing suppressive therapy after treatment.

Aortic dissection and ruptures in adult congenital heart disease in Texas from 2009 to 2019.

OBJECTIVES: Acute thoracic aortic dissection and rupture (TADR) has an incidence of 5-7 per 100 000-person years. Today, most children with congenital heart disease (CHD) survive to become adults with congenital heart disease (ACHD). This study evaluates TADR in patients with ACHD in a large, hospitalized patient population over 11 years to evaluate the incidence, risk factors and outcomes associated with TADR. METHODS: This was a retrospective review of the Texas Inpatient Discharge Data Set from 1 January 2009 to 31 December 2019. All non-trauma discharges of patients ≥18 years were included. ACHD discharges were identified by International Classification of Diseases, 9th edition (ICD-9)/10 diagnosis codes. TADR were identified using 2 definitions: TADR1 is an ICD-9/10 code for TADR, and TADR2 is TADR1 with an ICD-9/10 procedure code for aortic intervention. Descriptive, univariate and logistic regression statistics were used. RESULTS: A total of 22 154 664 eligible discharges were identified, of which 12 584 (0.06%) were TADR1 and a subgroup of 5699 (0.03%) were TADR2. CHD was more prevalent in TADR1 (0.2% vs 0.05%; P < 0.001) and TADR2 (0.3% vs 0.04%; P < 0.001). Adjusting for known TADR risk factors, CHD had an odds ratio of 1.69 (95% confidence interval: 1.09-2.63; P = 0.020) for TADR1 and an odds ratio of 1.69 (95% confidence interval: 0.99-2.88; P = 0.056) for TADR2. No in-hospital deaths were found in patients with CHD with TADR. CONCLUSIONS: ACHD discharges had a higher frequency of TADR versus the general population (0.9-1.2 vs 0.3-0.6 per 1000 discharges). There is an indication that CHD confers an increased adjusted odds of TADR. As the ACHD population continues to grow in number as well as age, it will be important to continue to assess the risk of TADR from CHD and how traditional risk factors impact this risk.

Measuring what matters: identifying assessments that reflect learning on the core surgical clerkship.

Purpose: There are various assessments used during the core surgical clerkship (CSC), each of which may be influenced by factors external to the CSC or have inherent biases from an equity lens. In particular, the National Board of Medical Examiners' Clinical Subject Exams ("Shelf") is used heavily and may not reflect clerkship curriculum or clinical learning. Methods: This is a retrospective review of medical student characteristics and assessments during the CSC from July 2017-June 2021. Assessment methods included: subjective Clinical Performance Assessments (CPA), Shelf, Objective Structured Clinical Examinations, and a short-answer in-house examination (IHE) culminating in a Final Grade (FG) of Honors/Pass/Fail. A Shelf score threshold for Honors was added in academic years 2020-2021. Descriptive, univariate, and multivariable logistic and linear regression statistics were utilized. Results: We reviewed records of 192 students. Of these, 107 (55.7%) were female, median age was 24 [IQR: 23-26] years, and most were White/Caucasian (N = 106, 55.2%). Univariate analysis showed the number of Exceeds Expectations obtained on CPA to be influenced by surgical subspecialty taken (p = 0.013) and academic year (p < 0.001). Shelf was influenced by students' race (p = 0.009), timing of CSC before or after Internal Medicine (67.9 ± 7.3 vs 72.9 ± 7.1, p < 0.001), and Term taken (increasing from 66.0 ± 8.7 to 73.4 ± 7.5, p < 0.001). IHE scores did not have any external associations. After adjustment with multivariable logistic and linear regressions, CPA and IHE did not have external associations, but higher scores were obtained on Shelf exam in Terms 3, 5, and 6 (by 4.62 [95% CI 0.86-8.37], 4.92 [95% CI 0.53-9.31], and 7.56 [95% CI 2.81-12.31] points, respectively. Odds of FG honors were lower when Shelf threshold was implemented (OR 0.17 [95% CI 0.06-0.50]), and increased as students got older (OR 1.14 [95% CI 1.01-1.30]) or on specific subspecialties, such as vascular surgery (OR 7.06 [95% CI 1.21-41.26]). Conclusions: The Shelf is substantially influenced by temporal associations across Terms and timing in relation to other clerkships, such as Internal Medicine. An IHE reflective of a clerkship's specified curriculum may be a more equitable summative assessment of the learning that occurs from the CSC curriculum, with fewer biases or influences external to the CSC. Supplementary Information: The online version contains supplementary material available at 10.1007/s44186-022-00047-8.

Re-Operative Aortic Arch Surgery in a Contemporary Series.

Re-operative aortic arch operations (REDO) following previous cardiac surgery are challenging procedures associated with significant morbidity and mortality. We investigated post-operative outcomes for patients undergoing REDO and identified risk-factors for mortality in a contemporary series. From 1/2005-6/2018, 365 consecutive patients at an academic center underwent REDO: 257 HEMIARCH and 108 COMPLETE arch (45 stage I elephant trunk, 63 total arch) replacements. Outcomes included mortality and major adverse events. Long-term survival was determined with Kaplan-Meier analysis, and risk-factors for mortality were assessed with Cox proportional hazards regression. Operative mortality for the entire cohort was 6.8%, and rates of stroke, cardiac arrest, and renal failure were 6.0%, 7.4%, and 10.4%. Compared to HEMIARCH, COMPLETE patients had an increased incidence of renal failure requiring dialysis (15.7% vs 8.2%, p = 0.031) and re-exploration for bleeding or delayed chest closure (19.4% vs. 11.7%, p = 0.051). Although operative mortality was similar in both cohorts, long-term follow-up mortality (38.0% vs 26.8%, p = 0.047) was higher among COMPLETE vs. HEMIARCH. Predictors of overall mortality among all-comers undergoing REDO included older age, low body surface area, endocarditis, ejection fraction <30%, emergent status of operation, extended cardiopulmonary bypass duration, intra-aortic balloon pump use, and a more extensive arch operation. Previous aortic surgery was not a risk-factor for mortality. Among all-comers undergoing REDO, survival was 81.4% at 1 year, 66.7% at 5 years, and 56.4% at 10 years of follow-up. While early postoperative outcomes are similar among HEMIARCH and COMPLETE, a more extensive arch-replacement is an independent risk-factor for overall mortality in REDO. Using appropriate clinical indications in the current era, REDO remains a viable option for selected patients.

Acquired Sinus of Valsalva Fistula and Turner Syndrome.

A 21-year old woman with a history of Turner syndrome presented with a diastolic heart murmur, dizziness, dyspnea, and intermittent chest pain. Preoperative imaging revealed a fistula from the right sinus of Valsalva into the right atrium. Turner syndrome is associated with both aortopathy and congenital heart malformations. Acquired sinus of Valsalva fistula is a rare disorder, and this report describes its presence in association with Turner syndrome.

Benchmarking Outcomes: Reoperation for Aortic Valve Patient-Prosthesis Mismatch.

BACKGROUND: Surgical reoperations for symptomatic aortic valve patient-prosthesis mismatch (PPM) are complex. The purpose of this research was to investigate the clinical and echocardiographic outcomes of patients who underwent reoperation for correction of symptomatic PPM after prior aortic valve replacement. METHODS: An institutional review identified 60 patients from 2004 to 2018 who underwent reoperative aortic valve replacement for PPM without structural valve degeneration. Univariate analyses were conducted to evaluate risk factors for perioperative mortality. RESULTS: Median patient age was 62.8 (interquartile range, 48.3-68.7) years, and mean body mass index was 29.2 ± 6.6 kg/m2. Thirty-nine (66%) patients underwent a first-time reoperation, and 18 (30.5%) underwent a second-time reoperation. The median interval between initial operation to reoperation for the treatment of PPM was 89 months. Thirty-four (56.7%) patients underwent aortic root replacement, while the remainder (43.3%) underwent aortic valve replacement. Additional procedures included replacement of the ascending aorta with or without aortic arch in 26 (43.3%) patients and coronary artery bypass grafting in 7 (12%) patients. Operative mortality and the rates of stroke and renal failure were 5.0%, 3.3%, and 5.0%, respectively. Echocardiographic follow-up was available in 64.9% of patients at a mean follow-up of 36.5 months. Preoperative mean pressure gradients were 32.1 ± 16.0 mm Hg and mean aortic valve area was 0.8 ± 0.3 cm2, and both improved to 6.6 ± 4.2 mm Hg and 2.3 ± 0.7 cm2 (P < .001). CONCLUSIONS: Reoperative surgery for PPM is complex but may be performed with good outcomes and low mortality in experienced centers. These data provide excellent clinical and hemodynamic benchmarks for the treatment of PPM in the current era of valve-in-valve transcatheter aortic valve replacement.

Anomalous Aortic Origin of a Coronary Artery: Surgical Emergency?

Anomalous aortic origin of a coronary artery is a congenital condition associated with sudden cardiac death. There are no current recommendations for the acute management and urgency for this patient population. This manuscript describes and discusses two patients who presented with an acute coronary event and needed emergent intervention despite initial clinical stabilization and improvement.

Rare Presentation of Endocarditis and Mycotic Brain Aneurysm.

Bartonella endocarditis can be an elusive diagnosis. The clinical manifestations can vary and, at times, include multiorgan involvement. This case report describes 2 patients who presented with multiorgan failure, cerebral mycotic aneurysms, and valvular endocarditis secondary to Bartonella infection. The complex diagnosis, decision making, and surgical management are described.

Sickle Cell-Related Complications in Patients Undergoing Cardiopulmonary Bypass.

BACKGROUND: We aimed to describe our experience with patients with sickle cell trait (SCT) and undergoing surgery on cardiopulmonary bypass (CPB). METHODS: Data on all patients with SCT or sickle-α thalassemia who underwent surgery on CPB were collected (1996-2017). RESULTS: Overall, 46 patients were included, 37 (80%) had SCT and 9 (20%) had sickle-α thalassemia. A total of 4 (9%) developed a potential sickle cell-related complication. Patients with sickle cell-related complications were significantly older (median 14 years vs 14 months, P = .037) and heavier (median 54 kg vs 9 kg, P = .041). Complications occurred, although without statistical significance, in patients who underwent longer median CPB times (249 minutes vs 137 minutes, P = .069), lower median temperature (31.7 °C vs 33.3 °C, P = .094), and a higher percentage underwent deep hypothermic circulatory arrest (50% vs 7%, P = .053). A total of 30 (65%) patients underwent exchange transfusion (ET) pre-bypass. Patients who underwent ET were significantly older (median 4 years vs 7 months, P = .003) and heavier (median 16 kg vs 6 kg, P = .015) than patients who did not undergo ET. The incidence of complications was comparable between patients who underwent ET (10%) and those who did not (6%). CONCLUSIONS: In this retrospective, single-center study, it has been shown that cardiac surgery requiring CPB in patients with SCT and sickle-α thalassemia had a low risk for sickle cell-associated complications. In this cohort of patients, older age, longer CPB times, lower median temperature, and the utilization of deep hypothermic circulatory arrest appear to play an important role in the development of complications.

Interrupted Aortic Arch in a Patient with Patient-Prosthesis Mismatch after Aortic Valve Replacement.

This case presents a patient who underwent aortic valve replacement and presented 13 years later with high gradients across the prosthesis, mitral insufficiency, and severe systemic hypertension. Her preoperative workup led to the diagnosis of an interrupted aortic arch Type A. Her surgical management included an initial procedure to repair the interruption, and 11 months later after resolution of her hypertension, a second surgery, which included the Ross procedure and mitral valve repair.